The Long Pain Road
For Chris Nolze
By
Kathleen Sweeney
Chris Nolze was diagnosed with brain cancer in 2007 at the young age of twelve. It was his initial diagnosis and the first of his major medical health issues.
He received radiation treatment after they did a biopsy on the tumor and placed a shunt in his head. It seems that where the tumor was located there was a lot of spinal fluid built up in his skull that was not draining. It was only the beginning of a number of challenges to follow.
During and after the radiation treatment he wasn’t “presenting” as well as other patients. They were not sure what was going on. There were signs of other medical issues that kept coming up and they are finally now discovering that the medical issues he has been dealing with since that time “are related either directly or correlated, to the radiation treatment”.
“Ever since my cancer I’ve had over 20 surgeries; … the majority of them were considered to be brain surgeries. Between the trauma from the tumor and then the trauma from all of the surgeries and then the trauma of radiation treatment my nerves were put at high risk … My cranial nerve and my trigeminal nerve just happened to be in that mix. “
When Chris had his wisdom teeth removed in 2012 he had complications that resulted in dry sockets. He was in pain but both he and his doctor thought it was due to the surgery. But it didn’t go away. Finally his dental surgeon suggested that they look into trigeminal neuralgia. It was a stretch since he did not fit the “norm” being a young boy with pain on both sides that was constant.
Neither Chris nor his parents had ever heard of trigeminal neuralgia. But since they had tried every possible medication at least twice and then in combinations and none of them gave him any relief they sought out his neurologist.
His neurologist and oncologist both thought it was highly “unlikely” that he had TN since he wasn’t showing the “proper” symptoms. Besides, it “just wasn’t possible”! For the next six months his parents took him all over this country looking for a doctor who would have another diagnosis.
Eventually they found Dr. Ben Carson in Maryland who tested the nerve on the right side of his face with a Rhizotomy treatment, which is the numbing or affecting of the nerve directly to see if it is the cause. “It helped the pain completely! … It was definitely a breath of fresh air.” It was the first relief he had had in six long months. “But I still did have constant pain on my left side.“
Shortly thereafter Dr. Carson scheduled Chris for an MVD (micro vascular decompression) on the other side, the left side. As he was recovering from that surgery the Rhizotomy “gave out” on the right side so they scheduled him for another MVD on the right side this time.
The pain was relieved but the incision would not close. They discovered that the skull bone itself was infected. They brought in a plastic surgeon to remove a piece of the skull about the size of a quarter. He placed a mesh plate over the opening created and then pulled the muscle up and over it (some of which had also been removed.) Eventually the incision closed properly.
Chris was pain free for about a year.
Then both sides came back and at about the same time. And around this same time he also contracted a rare case of intractable singultus (out of control hiccups). His is only the third case in medical history with this particular form. “They were speeding up on a daily basis and it got to the point where I would be doing over 200 hiccups in a minute! And it just kept getting faster and faster.”
It was a chiropractor, thinking outside the box, who put pads near his mesh plate and connected a tens unit to it. When it was turned on the hiccups stopped “instantaneously!”
So began another search for another doctor, this time with a tens unit connected to his head! Chris and his parents traveled the entire country again, finally finding Dr. Mark Linskey in California. He was actually Chris’ last and final hope of not having to live the rest of his life with a tens unit taped to his head.
“In every sense of the word, Dr. Linskey saved my life” says Chris. An MRI showed compressions on both his brain stem and on his facial nerves. Dr. Linskey thought the compressions on Chris’ brain stem might be the cause of the hiccups. While performing a repeat MVD on the right side of his face he tried lifting two major arteries off the brain stem. The hiccups stopped.
Out of curiosity and for research sake, Dr. Linskey released them and allowed them to go back where they were and the hiccups started back up again. When he lifted the arteries, they stopped. This confirmed that the cause of the hiccups was finally found! Since that surgery his right side has been not only TN pain free but he hasn’t had an abnormal case of hiccups since.
Once Chris recovered from that surgery they did a repeat MVD for trigeminal neuralgia on the left side. That was in 2014 and he is still pain free to this day!!
Although he cannot do any activities that might cause trauma to his head and has to avoid all sports, Chris is able to work part time. He is also studying online for his college degree, which he plans to receive this year.
In that same year of 2014, he joined the Young Patients Committee of the Facial Pain Association. It is a small group of less than 10 people who are supportive of anyone with facial pain under the age of 40. They promote awareness and help research for the really young suffering from all types of facial pain (not just trigeminal neuralgia.)
Chris also started his own foundation after his bout with cancer. He wants to give back to the community who “gave to my family and myself as we were going through everything. … I also wanted to raise funds for medical research for a lot of these radical conditions out there, especially trigeminal neuralgia.
“I focus on raising funds for research … and I have actually raised and donated $692 from my foundation to the Facial Pain Research Foundation” by selling teal colored t-shirts with his foundation logo on the front. A school friend of his hand knits teal scarves and he sells them also. All proceeds are donated to FPRF.
Chris does all he can to get the word out and to bring awareness. He has been recognized by the mayor of his town in Tom’s River, New Jersey, a couple of times already, with township proclamations that show that his foundation’s efforts are being recognized. He received one in 2016 from Governor Christy proclaiming October 7th Trigeminal Neuralgia Awareness Day! Since then, Chris has also received multiple proclamations from the town council recognizing every time the foundation helps out or works to spread awareness.
Chris volunteered that he wouldn’t change a thing if he could. “It helped sculpt me into the man I always wanted to be, that I was meant to be, and even if I had the opportunity to somehow stop everything from happening and go back in time and just live it out as ‘normal’ I wouldn’t change anything.” He is very insightful for his age. And very brave I might add.
When asked for what advice he would give to anyone new to the pain of trigeminal neuralgia he replied “The number one piece of advice I would give is to not give up and to not lose hope because even though you may be faced with a hundred people saying ‘No. It’s not possible.’ that doesn’t mean that there is absolutely no help out there for you.” And “Let your heart kick in and just say ‘no I’m not going to give in until I get the answers - until I get the proper treatment. I know something wrong is going on right now.’”
He added “It made me grow up much faster than I anticipated but in a way it also helped me.” I can vouch for that. Chris certainly seems a whole lot older and wiser than any 22 year-old I’ve ever known!
CODA Joins Facial Pain Research Foundation
International Scientific Consortium
By Michael Pasternak
I am excited to say on New Years Day 2018, The Facial Pain Research Foundation (FPRF) has six Foundation and two corporate funded scientific research projects actively working to find the cure for trigeminal neuralgia and related neuropathic pain. The scientists working on these projects are members of our International Scientific Research Consortium. In the past this World Famous WebNewspaper has printed numerous stories about the Foundation funded projects (see Dr. Douglas Andersons 2017 Report). In October, 2017 the FPRF announced that Neurona Inc. (a corporate funded research project joined the FPRF Scientific Consortium. Neurona is focused on the discovery and development of cell-based therapies to treat intractable neurological diseases including TN and related neuropathic pain. Today the Foundation is pleased to announce that CODA, another corporate funded scientific project, is joining the FPRF International Research Consortium.
CODA Biotherapeutics is a San Francisco based pre-clinical stage biotechnology company developing a novel therapeutic platform for the treatment of chronic pain and other severe neurological disorders. CODA’s innovative platform relies upon a gene therapy approach to modulating aberrant neural activity, which CODA’s co-founders -- a team of forward-thinking biotechnology entrepreneurs, scientists, and clinicians -- believe will provide relief to patients suffering from intractable neuropathic pain such as that associated with trigeminal neuralgia and establish a new treatment paradigm for severe neurological disorders. The entire CODA team enthusiastically welcomes the opportunity to join the Facial Pain Research Foundation’s Scientific Consortium in developing new treatment options for patients with trigeminal neuralgia.
Building upon the groundbreaking work of the company’s founders and Scientific Advisory Board members, several of whom have been associated with FPRF, CODA is using advanced synthetic biology approaches to engineer novel neurotransmitter receptors that respond selectively to non-addictive and non-opioid-based small molecules. These engineered neurotransmitter receptors are delivered to pain-signaling neurons using viral vectors by standard of care neurosurgical procedures. Hyperactive target neurons that express the receptor, including those in the trigeminal ganglia, can then be controllably silenced by oral administration of the safe small molecule. Because the treatment molecule exclusively activates the receptor, it is possible to achieve highly selective blockade of the critical pain signals from reaching the brain. This effectively opens the “therapeutic window”, reducing the potential for adverse side effects. Preclinical studies in multiple neuropathic pain models have demonstrated that this technology provides robust, durable, tunable, and reversible pain control, without the side effects experienced with existing small molecule therapies (e.g. opioids, anticonvulsants, tricyclic antidepressants) or neurosurgical procedures (e.g. ablation, neurostimulation implants). CODA anticipates that this technology will be used to manage intractable chronic pain across a multitude of conditions, including trigeminal neuralgia, and it will be applied broadly to other peripheral and central nervous system disorders in the future.
First Published Sept 23, 2014
We’ve come a long way! Join us and make our journey shorter!
By Roger Levy
Back in 1990, when the Trigeminal Neuralgia Association was first organized, its growth and development depended on the volunteer founders and some leading neurosurgeons who were concerned that little was being done to help patients understand their condition and gain access to specialists who could treat their condition. The Internet had yet to develop and getting the word out was managed through support groups started by volunteer patients who had become acquainted with the Association.
As time went on and the number of patients who looked to the Association for information and support began to grow so did the association’s awareness of the limitations which affected the medical and surgical options available as treatment. The drugs, after all, do nothing but dull the pain and most of the surgical procedures involve permanent damage to the nerve and that damage can often result in increased and, sadly, inoperable pain if the procedure fails. Gamma Knife treatment which involves radiation is promoted by many centers as non-invasive and yet there are too many patients who have increased pain where the radiation treatment failed. Even microvascular decompression which has a high success rate and involves no permanent damage to the nerve has its limitations because it involves a hospital stay and general anesthetic, for which some patients are not candidates either through choice or some other medical condition.
Still, over the years, the Association has helped many tens of thousands of patients to gain relief and regain a good quality of life. This is an important and continuing part of the Association’s mission on which it continues to deliver. But what of tomorrow’s patients or those today with unresolved pain? What’s to be done about them?
That is a question which the Association began to address when your Founding Trustees, Michael Pasternak, Mike Hirsch and I were members of the Association’s Board. Faced with that challenge, we embarked on an ambitious plan to establish a facial pain research program at the McKnight Brain Institute at the University of Florida, where Doug Anderson, Foundation trustee, was then the Executive Director. However, raising sufficient funds for research and meeting other parts of the Association’s mission was too great a challenge. Thus we chose to establish The Facial Pain Research Foundation.
That was over two years ago and look how far we have come! In a short period of time, The Facial Pain Research Foundation has raised over $1,300,000 and has provided funding for 4 separate research projects intended to find a permanent end to the pain of trigeminal neuralgia. Now, as word gets out across the internet via our Web Newspaper, people are contacting us from all over the world to ask “How can we help?” - Something unimaginable in 1990. When I was diagnosed with TN in that year, there were few places to turn. My neurologist could only recommend Tegretol or, when I proved allergic, acupuncture. Surgery, he warned me, was to be avoided at all costs. It was not until 1997 that the internet lead me to the Association and to a quick path to relief through microvascular decompression. Even with all those years of pain, though, I count myself lucky now that I am pain and medication free. Not every patient enjoys that success and for them and the patients who have yet to come, the work of the Foundation is imperative.
If you are reading this, chances are you are doing so because of the reach of the internet and, so rather like the support groups of the ‘90s, you have the opportunity to support our cause and bring our journey to a successful conclusion more swiftly. You can help in many ways as you can read elsewhere in our Newspaper, including making a financial contribution. Contributions are now coming to us almost daily with words of support and encouragement. No amount is too small to win our gratitude or to help.
We have world class scientific investigators enthusiastically working on behalf of facial pain patients, without national or other boundaries. They are our researchers “sans frontieres” and the benefits of their research will similarly lack borders. We are a community brought together by common cause. Be part of that community and help us to achieve our goals.
First Published June 13, 2014
Dr. Mark Linskey is a Professor of Neurological Surgery currently practicing at the University of CA, Irvine, in Orange County, CA (http://www.faculty.uci.edu//profile.cfm?faculty_id=5322). He trained in microvascular decompression (MVD) for 7 years with Peter Jannetta and in Gamma Knife stereotactic radiosurgery (GKSR), glycerol rhizotomy and radiofrequency lesioning for 7 years with L Dade Lunsford at the first US gamma Knife unit at the University of Pittsburgh. He has continuously served on the Medical Advisory Board of TNA - The Facial pain association since 2003; currently serving as their Western Regional Director. In practice and out of training for more than 20 years, he published the first prospective cohort comparative clinical trial of MVD versus GKSR for typical trigeminal neuralgia and is an internationally-recognized expert, and experienced surgical subspecialist, in trigeminal neuralgia. He is currently the American College of Physicians (ACP) Trigeminal Neuralgia module editor for their physician point of care decision support tool "Smart Medicine" (formerly PIER - Physician Information & Education Resource). He is also the Co-Editor of the Trigeminal Neuralgia module for the rigorous evidence-based medicine periodic systematic review tool and physician decision-support resource, "Clinical Evidence", published by the BMJ (British Medical Journal) for the British Medical Association.
Facial Pain The Research Imperative
By Mark E. Linskey, M.D.
From a twisted and perverse perspective, among facial pain patients, patients with classical trigeminal neuralgia (TN), are comparatively fortunate. We at least have surgical treatment options with reasonable chances for successful pain relief when current medical therapy fails. Yet even for TN patients, not all patients fare well. While in expert and experienced surgical hands, microvascular decompression (MVD) can lead to durable complete pain relief in about 80% of patients, this still leaves one in five TN patients still suffering. Patients seeking care in the general medical community are not so fortunate. MVD results can vary significantly surgeon-to-surgeon. Empiric experience over the last 20 years in my practice, would suggest that the success rate among less experienced and expert surgeons might run as low as 50%.
For those patients with multiple sclerosis-related TN, or who fail MVD, their plight can be improved with palliative destructive procedures directed towards the trigeminal nerve [stereotactic radiosurgery (SR, radiofrequency lesioning (RFL), glycerol rhizotomy (GR), and balloon compression (BC)]. However, these procedures serve best as temporizing measures, with up to 50% of patients with classical TN recurring within five years of the procedure. Each palliative destructive procedure runs the risk of facial numbness with its associated risk of new de-afferentation pain from nerve damage, and these risks accumulate and compound as destructive procedures are repeated. Thus overall, across the U.S., even for patients with classical TN, surgical intervention may realistically not provide adequate or long-lasting relief in an estimated 30-50% of cases.
But what about those patients with other forms of facial pain? In 2008, after 18 years of existence, The Trigeminal Neuralgia Association (TNA) changed its name to TNA – The Facial Pain Association (TNA-FPA) in recognition of the fact that patients with facial pain other than classical TN also needed education, support and help too. These conditions include TN2, neuropathic facial pain, facial pain of obscure etiology, and anesthesia dolorosa, among many others. For these patients, MVD rarely leaves a patient pain-free. Furthermore, while palliative destructive procedures can, and do help, they not only are less successful than for classical TN patients, they may actually worsen the situation through increased de-afferentation, despite the best of surgical intentions. While surgical stimulator surgery can help these patients in very select cases, the overall percentage of patients enjoying significant and durable relief is disappointingly small. If we now broaden our inquiry to include all facial pain patients that seek help from TNA-FPA, it seems likely that only about half will achieve adequate or long-lasting relief from their suffering through surgery. Clearly surgery alone is not the answer for too many of our patients, friends, and family who suffer from facial pain.
The word imperative refers to something that is both necessary and of vital or crucial importance. It may also refer to the expression of a command. For facial pain syndromes new discovery and therapeutic breakthroughs through basic science research is an absolute imperative in the first sense and an imperative from our patients and their loved ones in the second sense.
We know that vascular compression is a necessary cause for the majority of classical TN patients. Yet up to 40% of normal living patients and up to 50% of patients studied at autopsy have these vascular contacts without having TN. Clearly vascular compression, while necessary, is not sufficient. The logical additional factor, that many have speculated about, likely involves additional predispositions related to genetics and/or the biology of myelin nerve insulation, axonal damage, or both. In my opinion, studies of deferential gene expression comparing classical TN patients to normal population controls carry the highest chance of uncovering potentially new fruitful areas for investigation and future therapeutic exploitation for patients with classical TN.
While the hoped for discoveries from studying differential gene expression in cases of classical TN might be more generalizable to help patients with other facial pain syndromes, this is by no means certain. Thus, simultaneous additional lines of research inquiry are also desperately needed. These include the urgent need for a better understanding of the neurochemistry and the psycho-biology of chronic neuropathic pain and the fast-track development of new agents for clinical testing. It includes studies into the biology of nerve repair and regeneration, through both pharmacological means as well as potential cellular therapies including stem cells. It also includes the need to study safe means of modulating pain perception at the brain level through brain stimulation technology.
While it is simple and conceptually satisfying to talk about the search for one “cure”, this may be an elusive goal given the heterogeneity and wide variety of facial pain syndromes suffered by patients seeking help through the TNA-FPA. More than one “cure” may be needed depending on the individual circumstances of each patient. Yet the quest for a “cure”, whether one or many, all still depend on basic science research advances which are expensive and which take years to develop, test and realize.
As a surgeon, I long for the day when we have therapies that can increase our MVD results to near 100% success, as well as offer hope and more predictable treatment success chances to our non-classical TN facial pain patients. While I am biased by my surgical background, I doubt that these advances will involve a new pill distributing an agent non-specifically throughout our body. I strongly suspect that they will involve therapies that will be most effective locally and selectively at the site of pathology or damage, either through direct open surgical application, or needle or electrode delivery into the trigeminal nerve root, ganglia, or brain. Thus surgery will likely be an important component of any success realized through our research efforts.
If we wish the state of care for patients with facial pain to improve from the levels currently available, we are indeed faced with a research imperative. The need is great and the costs are considerable. The best way that most can help is through generous donation and ongoing, sustained, support for efforts such as those fostered by our own Facial Pain Research Foundation http://www.facingfacialpain.org/ . We need everyone’s help and support to drive this mission forward and ultimately succeed.
https://today.duke.edu/2017/11/why-head-and-face-pain-causes-more-suffering
Why Head and Face Pain
Causes More Suffering
Sensory neurons in the head and face tap directly into the brain’s
emotional pathways
November 13, 2017
Hate headaches? The distress you feel is not all in your -- well, head. People consistently rate pain of the head, face, eyeballs, ears and teeth as more disruptive, and more emotionally draining, than pain elsewhere in the body.
Duke University scientists have discovered how the brain's wiring makes us suffer more from head and face pain. The answer may lie not just in what is reported to us by the five senses, but in how that sensation makes us feel emotionally.
The team found that sensory neurons that serve the head and face are wired directly into one of the brain’s principal emotional signaling hubs. Sensory neurons elsewhere in the body are also connected to this hub, but only indirectly.
The results may pave the way toward more effective treatments for pain mediated by the craniofacial nerve, such as chronic headaches and neuropathic face pain.
“Usually doctors focus on treating the sensation of pain, but this shows the we really need to treat the emotional aspects of pain as well,” said Fan Wang, a professor of neurobiology and cell biology at Duke, and senior author of the study. The results appear online Nov. 13 in Nature Neuroscience.
Pain signals from the head versus those from the body are carried to the brain through two different groups of sensory neurons, and it is possible that neurons from the head are simply more sensitive to pain than neurons from the body.
But differences in sensitivity would not explain the greater fear and emotional suffering that patients experience in response to head-face pain than body pain, Wang said.
Personal accounts of greater fear and suffering are backed up by functional Magnetic Resonance Imaging (fMRI), which shows greater activity in the amygdala -- a region of the brain involved in emotional experiences -- in response to head pain than in response to body pain.
“There has been this observation in human studies that pain in the head and face seems to activate the emotional system more extensively,” Wang said. “But the underlying mechanisms remained unclear.”
To examine the neural circuitry underlying the two types of pain, Wang and her team tracked brain activity in mice after irritating either a paw or the face. They found that irritating the face led to higher activity in the brain’s parabrachial nucleus (PBL), a region that is directly wired into the brain’s instinctive and emotional centers.
Then they used methods based on a novel technology recently pioneered by Wang’s group, called CANE, to pinpoint the sources of neurons that caused this elevated PBL activity.
“It was a eureka moment because the body neurons only have this indirect pathway to the PBL, whereas the head and face neurons, in addition to this indirect pathway, also have a direct input,” Wang said. “This could explain why you have stronger activation in the amygdala and the brain’s emotional centers from head and face pain.”
Further experiments showed that activating this pathway prompted face pain, while silencing the pathway reduced it.
“We have the first biological explanation for why this type of pain can be so much more emotionally taxing than others,” said Wolfgang Liedtke, a professor of neurology at Duke University Medical Center and a co-author on Wang’s paper, who is also treating patients with head- and face-pain. “This will open the door toward not only a more profound understanding of chronic head and face pain, but also toward translating this insight into treatments that will benefit people.”
Chronic head-face pain such cluster headaches and trigeminal neuralgia can become so severe that patients seek surgical solutions, including severing the known neural pathways that carry pain signals from the head and face to the hindbrain. But a substantial number of patients continue to suffer, even after these invasive measures.
“Some of the most debilitating forms of pain occur in the head regions, such as migraine,” said Qiufu Ma, a professor of neurobiology at Harvard Medical School, who was not involved in the study. “The discovery of this direct pain pathway might provide an explanation why facial pain is more severe and more unpleasant.”
Liedtke said targeting the neural pathway identified here can be a new approach toward developing innovative treatments for this devastating head and face pain.
This research was supported by grants from the National Institutes of Health (DP1MH103908, F31 DE025197-03, K12DE022793). Dr Liedtke is also supported by the Facial Pain Research Foundation (Gainesville FL).
CITATION: "A Craniofacial-Specific Monosynaptic Circuit Enables Heightened Affective Pain," Erica Rodriguez, Katsuyasu Sakurai, Jennie Xu, Yong Chen, Koji Toda, Shengli Zhao, Bao-Xia Han, David Ryu, Henry Yin, Wolfgang Liedtke and Fan Wang. Nature Neuroscience, Nov. 13, 2017. DOI: 10.1038/s41593-017-0012-1
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